%0 Journal Article
%T Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans
%A Emanuela Palmerini
%A Marco Gambarotti
%A Eric L Staals
%A Licciana Zanella
%A Gabriela Sieberova
%A Alessandra Longhi
%A Marilena Cesari
%A Stefano Bonarelli
%A Piero Picci
%A Pietro Ruggieri
%A Marco Alberghini
%A Stefano Ferrari
%J Clinical Sarcoma Research
%D 2012
%I BioMed Central
%R 10.1186/2045-3329-2-4
%X DFSP and fibrosarcomatous-DFSP (FS-DFSP) patients referred to our institute between 1982 and 2009 were identified. Fibrosarcomatous changes, expression of CD34 and apolipoprotein-D were evaluated.40 patients, (median age 43 years, 55% males) were identified. Tumor was located in the limbs in 60%, in the trunk in 40%. Thirty-seven patients had localized and 3 had metastatic disease. Thirteen (32%) patients were FS-DFSP. All but one underwent surgery with adequate surgical margins in 72%. 7 FS-DFSP received also radiotherapy (RT). Chemotherapy was administered to 3 patients with FS-DFSP. With a median follow-up of 49 months, the 5-OS was 90%. Local recurrence rate was 23%: 42% FS-DFSP, 15% DFSP. Metastases developed in three FS-DFSP patients. The 5-year EFS was 70% in localized patients. Histology (DFSP 75% vs. FS-DFSP 52%, p = 0.002), surgical margins (adequate 74% vs. inadequate 55%, p = 0.02), site (limb 47% vs. trunk 100%), CD34 expression (CD34 positive: 70% vs. CD34 negative: 33%, p = 0.05), and apolipoprotein-D expression (Apo-D positive: 73% vs. Apo-D negative: 33%, p = 0.02) influenced the 5-year EFS, whereas sex, use of RT or number of previous surgical treatments did not.Patients with DFSP have a high survival probability. Site, adequate surgical margins, presence of the fibrosarcomatous component, lack of CD34 expression and apolipoprotein-D influence outcome.Dermatofibrosarcoma protuberans (DFSP) is a low grade malignant mesenchymal tumor that typically arises in the dermis of the trunk and proximal extremities [1]. DFSP represents 1 to 6% of all soft tissue sarcomas (STS) [2,3] and its frequency of detection slowly has increased over time [4]. DFSP is characterized by latency of initial diagnosis, slow infiltrative growth and a high rate of local recurrence if not adequately treated. Death due to metastatic disease is very rare (< 5%) [5]. Histologically, DFSP is usually characterized by uniform spindle shaped cells with elongated neuroid nuclei, prolife
%K dermatofibrosarcoma protuberans
%K soft tissue sarcoma
%K Apolipoprotein-D
%K CD34
%U http://www.clinicalsarcomaresearch.com/content/2/1/4